Hemophilia is an inherited disease in which your blood does not clot. People with hemophilia lack or have low levels of one of two blood-clotting substances, known as factor VIII and factor IX. As a result, they may bleed for a long time after an injury. They may also experience internal bleeding, especially in the joints. There are two types of hemophilia -- type A and type B. Hemophilia is the most common inherited bleeding disorder. About 17,000 people in the U.S. have the condition.

Alternative Names of Hemophilia are: Christmas Disease, Factor IX Hemophilia.

Complications of Hemophilia

The list of complications that have been mentioned in various sources for Hemophilia includes:

• Antibodies against transfused clotting factors.
• Arthritis from bleeding in the joints.
• Arthropathy (chronic joint disease) from bleeding in the joints - The major cause of disability in hemophilia patients is chronic joint disease "arthropathy" - caused by uncontrolled bleeding into the joints.
• Hemorrhage - Life threatening hemorrhage is a constant risk.
• Fatal Hemorrhage - These bleeds ultimately result in severely impaired joints.

Causes of Hemophilia

Hemophilia is an inherited disorder that mostly affects men. Women rarely have the disease, but they are carriers of the condition and can pass it on to male children.

Signs & Symptoms of Hemophilia

People with hemophilia may have the following signs and symptoms:

• Joint pain and swelling, frequently in the knees and elbows
• Heavy bleeding, or bleeding that lasts a long time, from an accident or other injury
• A delay in the start of bleeding
• Large bruises
• Swelling under the skin and between muscles, with fever, skin discoloration, and pain
• Internal bleeding in the abdominal region, airway, or central nervous system
• Bleeding of the mouth and gums, tooth loss
• Blood in the urine
• Digestive problems
• Nosebleeds

Diagnosis of Hemophilia

• Health care providers usually diagnose hemophilia before birth through a blood test.
• After birth, the first sign of hemophilia is often deep and easy bruising as an infant is learning to crawl.
• For male children, heavy bleeding after circumcision may be the first sign of the condition.
• Your health care provider may diagnose cases of mild hemophilia even later, after a tooth extraction or following surgery.
• During a physical examination, your health care provider will take a family history and check for deep bleeding, muscle spasms, limited joint motion, joints that are warm and enlarged, and bruises.
• Blood tests can show whether clotting factors are low or missing.
• Your health care provider may request computed tomography (CT) scans and magnetic resonance imaging (MRI) to find bleeding in the joints.

Treatments of Hemophilia

The list of treatments mentioned in various sources for Hemophilia includes the following list. Always seek professional medical advice about any treatment or change in treatment plans.

1. Transfusions.
2. Home intravenous transfusions.
3. Transfusion treatment strategies.

• On-demand infusion - the traditional use of infusion only when there are bleeding symptoms.
• Periodic infusion (prophylaxis) - a preventive ongoing method that reduces complications but is costly and more involved.

4. Clotting factor therapy - the use of replacements for the missing blood products by transfusion of concentrated clotting factors from blood donors.

• Factor VIII concentrates
• Factor IX concentrates

5. Special treatment required prior to surgery.
6. Special treatment required prior to dental procedures.
7. Special treatment required for any injury.

Prevention of Hemophilia
Hemophilia cannot be cured. People with hemophilia should take the following precautions:

• Avoid taking aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs).
• Get vaccinated (including infants) with the hepatitis B vaccine.
• Administer factor VIII or IX (see below) on a regular basis, to help prevent bleeding and joint damage.
• Avoid circumcising male infants of women known to be carriers until the baby has been tested for hemophilia.
• Avoid circumcising male infants of women known to be carriers until the baby has been tested for hemophilia.
• Carry information at all times identifying the person as someone with hemophilia.

When to seek Medical Advice

Certain bleeds require medical attention, including those injuries affecting:

• the central nervous system - any suspected trauma to the head, neck, or back
• the face, including the eyes and ears
• the throat or another portion of the airway
• the gastrointestinal tract (which might produce signs such as bright red or black blood in your child's stool)
• the kidneys and urinary tract (if you find blood in the urine, this may require treatment and bed rest)
• the iliopsoas muscle in the trunk (which might produce signs that mimic a hip or abdominal bleed, including lower abdominal/groin or upper thigh pain, an inability to raise the leg on the affected side, and a feeling of relief when contracting or flexing that side of the body)
• the genital area
• the hips or shoulders (these can be complicated bleeds because they involve the rotator joints)
• large muscle compartments, such as the thighs

If the bleed requires going to the emergency room, make sure your child is treated at a hospital that has experience treating hemophilia. Any injury affecting the brain or any part of the central nervous system or a vital organ should be treated as an emergency and you should get medical assistance immediately.